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SDHB Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Defects in SDHB are a cause of susceptibility to pheochromocytoma (PCC). A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent. Defects in SDHB are the cause of paragangliomas type 4 (PGL4). A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas are most commonly located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear. Defects in SDHB are a cause of paraganglioma and gastric stromal sarcoma (PGGSS); also called Carney-Stratakis syndrome. Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance. Defects in SDHB are a cause of Cowden-like syndrome (CWDLS). Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus. Belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC; Energy Metabolism - oxidative phosphorylation; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 1p36.13
Cellular Component: mitochondrial inner membrane; mitochondrial membrane; mitochondrial respiratory chain complex II; mitochondrion; nucleoplasm; plasma membrane
Molecular Function: 2 iron, 2 sulfur cluster binding; 3 iron, 4 sulfur cluster binding; 4 iron, 4 sulfur cluster binding; protein binding; ubiquinone binding
Biological Process: aerobic respiration; tricarboxylic acid cycle
Disease: Cowden Syndrome 2; Gastrointestinal Stromal Tumor; Paraganglioma And Gastric Stromal Sarcoma; Paragangliomas 4; Pheochromocytoma
Reference #:  P21912 (UniProtKB)
Alt. Names/Synonyms: DHSB; FLJ92337; Ip; Iron-sulfur subunit of complex II; PGL4; SDH; SDH1; SDH2; SDHB; SDHIP; Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
Gene Symbols: SDHB
Molecular weight: 31,630 Da
Basal Isoelectric point: 9.03  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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